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Isolated Pericardial Amyloidosis

Imaging findings

Chest CT and MRI in a 70-year-old man demonstrate circumferential pericardial calcification, chronic bilateral pleural effusions, tubular ventricles, and dilatation of the inferior vena cava, consistent with constrictive pericarditis. There is dense, cloudy pericardial fluid that exhibits a contrast-fluid level on CT. Pericardial biopsy confirms extensive amyloid deposition within the pericardium, with no evidence of myocardial amyloid involvement on MRI or histopathology.

Key takeaways

Pericardial amyloidosis causing isolated constrictive pericarditis without myocardial involvement is an extremely rare presentation of amyloid disease. It manifests with signs of constriction, including pericardial thickening, calcification, and a unique cloudy, protein-rich pericardial effusion. Surgical pericardial stripping is required to relieve constriction.

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